Leiomyosarcoma is a rare type of malignant tumor arising from the smooth muscle cells. They commonly affect the muscle cells of the abdomen, but can occur anywhere in the body. The other sites include skin, blood vessels, uterus, bladder, gastrointestinal tract, liver and pancreas. The uterus is, however, the most common site of leiomyosarcoma.
How Common is Leiomyosarcoma?
Leiomyosarcomas affect both the male and the female population. It is a soft tissue sarcoma affecting at least 15,000 people each year in the U.S. itself and constitutes for about 1% of all the adult cancers. However, uterine leiomyosarcomas usually account for 1-2% of all malignant tumors of the uterus, affecting at least 6 in every 1,000,000 women in the United States per year. Leiomyosarcoma is responsible for 7-11% of all soft tissue sarcomas. Soft tissue sarcomas mostly affect men more than women and have high occurrence in adults rather than children and adolescents.
The exact cause of leiomyosarcomas is not known except that the genetic factors could play a major role in their occurrence. The uncontrolled division of cells occurs when the tumor suppressor genes fail to function properly and the oncogenes undergo metastasis. It is said to be a defect in the DNA (deoxyribonucleic acid), which is the carrier of a person’s genetic code. These lead to the basis of cellular malignant transformations occurring in leiomyosarcomas.
The symptoms of leiomyosarcomas vary from person to person and also depend upon the extent, location and spread of the tumor. They include fever, nausea, vomiting of blood (hematemesis), sudden weight loss, lump or swelling, fatigue or weakness, discomfort in the abdomen, malaise (feeling of ill health) and pain may or may not be present. However, leiomyosarcomas in the gastroinstestinal tract may include stomach ache, melena (back tarry colored foul smelling stools), vomiting of blood. In the uterine leiomyosarcomas the symptoms are abnormal uterine bleeding, discharge from the vagina and altered bladder and bowel habits.
The leiomyosarcomas are aggressive tumors and they spread rapidly into other tissues and organs such as liver and lungs. If they are not diagnosed early they may lead to life threatening complications. Also the earlier the diagnosis is made, the better the prognosis of the tumor and less the recurrence.
Diagnosis of Leiomyosarcoma
The diagnosis of leiomyosarcoma is based on a thorough medical history of the patient. Routine tests of blood and investigations are done along with a biopsy to confirm the diagnosis. The CT scan, MRI and an ultrasound help in finding the location, extent and spread of the tumor, which will further help in the surgical treatment. FNAC (fine needle aspiration cytology) is usually done to confirm the diagnosis of leiomyosarcomas in which a fine thin long needle is passed into the tumor or mass and a small sample of the tissue is taken to study its histopathology.
Treatment of Leiomyosarcoma
The treatment for leiomyosarcoma depends on various factors such as location, stage and spread of tumor along with age, health and medical condition of the patient. There are three treatment modalities that are adopted for the management of leiomyosarcoma, which include surgical excision, radiation therapy and chemotherapy. Surgical excision is the mainstay of the treatment, which again depends on the location, size and extent of tumor. Radiation therapy is done postoperatively to treat the residual tumor. If surgery is not an option due to the location of tumor then radiation therapy is opted.
Chemotherapy can also be opted for along with surgery or radiation therapy for locally advanced, metastatic or recurrent cancer. However, radiation therapy and chemotherapy have only minimal success in cases of leiomyosarcoma. Since leiomyosarcoma is so rare, there has been no identifiable radiation or chemotherapy for it. They are still under investigation; Yondelis has been recently approved for leiomyosarcoma as a chemotherapeutic drug. The rarity of the disease makes its prognosis also poor and investigation and research stays limited in this field.